Introduction: Primary Pseudotumor cerebri (PPTC), also known as idiopathic intracranial hypertension, is clinically characterized by increased intracranial pressure in an alert and oriented patient, with no evidence of deformity or obstruction of the ventricular system on neuroimaging. Cerebrospinal fluid analysis is normal except for increased intracranial pressure at lumbar puncture, greater than the 90th percentile (28 cm of H2O) in the pediatric population. Papilledema may or may not be present. Headache is the most common presenting symptom of PPTC (84%) and is often described as daily, bilateral, frontal or retroocular. Obesity is a consistent risk factor for the development of PPTC. Association between body mass index (BMI) and risk of PPTC has been demonstrated.
Patient Presentation: A 16-year-old morbidly obese African Brazilian boy [weight: 133.6 kg; BMI: 44.1 kg/m2 (+3.83 standard deviations (SD)], stage Tanner 5, presented with bi-parietal, high intensity and pulsatile headaches. The headaches had progressively worsened over the last three months. They were occurring five times per week and were associated with nocturnal awakenings. There was partial improvement with common analgesics. He was not able to stand still or walk straight without falling during the headache episodes. Cranial computed tomography revealed no mass or anatomic abnormalities. Lumbar puncture showed an elevated opening intracranial pressure of 40 cm of H2O with normal contents. Ophthalmologic evaluation confirmed bilateral papilledema, normal visual acuity and absence of abducens nerve palsy. PPTC was diagnosed. The patient was started on acetazolamide (Diamox) q12h with partial improvement of his symptoms. However, after 3 months, he was still symptomatic. As he had already failed to lose weight after been enrolled in a medically supervised weight-loss program (composed of a multi-disciplinary team including nutritionist, physical therapist, psychologist and pediatric surgeon specialized in bariatric surgery), bariatric surgery was indicated. The patient underwent an uncomplicated laparoscopic sleeve gastrectomy. Ophthalmologic evaluation, performed 5 months after surgery, revealed normal visual acuity in both eyes and improvement of bilateral papilledema. Follow-up at 18 months showed a 67.5% excessive weight loss (weight: 94.5 kg and BMI: 31.2 kg/m2) and complete resolution of PPTC symptoms.
Conclusion: Our case shows that bariatric surgery may be a valid alternative approach for those morbidly obese adolescent patients with refractory symptoms. Our patient presented complete resolution of PPTC signs and symptoms and experienced a 67.5% excessive weight loss after surgery.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology